AB0676 One year progression of interstitial lung disease in connective tissue diseases. A descriptive study in a single tertiary center.
Authors: C. L. Garcia Gomez, L. Vega, O. Ibarguengoitia, M. Enjuanes, I. Calvo, D. Montero, J. M. Blanco, M. L. García Vivar, E. Galindez, A. R. Inchaurbe, I. Torre, I. Gorostiza, E. Cuande, M. E. Ruiz
Journal: Annals of the Rheumatic Diseases (2022)

Background Interstitial lung disease (ILD) in connective tissue diseases (CTD) is an important cause of morbidity and mortalitiy. Objectives To evaluate ILD in CTD (systemic sclerosis, myositis, Sjögren syndrome, rheumatoid arthritis, mixed connective tissue disease), sarcoidosis and interstitial pneumonia with autoimmune features and its progression in 12 months evaluated through high resolution computed tomography (HRCT) and pulmonary function test (PFT). Methods A retrospective single tertiary center cohort study in CTD-ILD outpatients seen between 2012 and 2021. Clinical, serological data, PFT and HRCT results were collected. ILD patterns were classified into: usual interstitial pneumonia (UIP), inconsistent UIP, nonspecific interstitial pneumonia (NSIP), fibrosing NSIP, organizing pneumonia, interstitial lymphoid pneumonia and associated to sarcoidosis. Progression of ILD was defined as: -&gt;10% decline in FVC in PFT. -&gt;15% decline in DLCO in PFT. -Progression of fibrosis in HRCT. IBM SPSS v23 was used for statistical analysis. Results 51 patients were collected. Baseline characteristics are shown in Table 1. Figure 1 shows ILD progression in 1 year. Table 1. Baseline characteristics. Sociodemographic characteristics Clinical features/affection– n (% ) Total 51 Raynaud 23 (45,1%) Female-n (%) 42 (82,4%) Skin 23 (45,1%) Age- years (mean) IQR) 56 (27-82) Ocular 13 (25,5%) Arthritis 16 (31,4%) Myositis 7 (13,7%) Renal 1 (2%) Esophagus 7 (13,7%) Hemathological 3 (5,9%) PHT 7 (13,7%) Smoking status- n (% ) Symptoms at ILD diagnosis - N (41) % Current 9 (17,6%) Cough 31,4% Former 13 (25,5%) Toracic pain 11,8% Never 29 (56,9%) Dyspnea 47,1% Comorbidities - n (% ) Pattern HRCT - n (% ) Diabetes mellitus 5 (9,8%) UIP 11 (21,6%) Ischaemic cardiopathy 3 (5,9%) Fib-NSIP 5 (9,8%) Hypertension 15 (29,4%) UIPincons 13 (25,5%) POCD 3 (5,9%) NSIP 15 (29,4%) OP 2 (3,9%) LIP 2 (3,9%) Sarcoidosis 3 (5,9%) Type of disease associated to ILD - n (% ) Immunosupression - n (% ) Systemic Sclerosis 14(27,5%) Methotrexate 20(39,2%) Sjögren’s Syndrome 7 (13,7%) Mycophenolate mofetil 16 (31,4%) Sarcoidosis 3 (5,9%) Hydroxychloroquine 19(37,3% Myositis 8 (15,7%) Cyclophosphamide 4 (7,8%) SLE 1 (2%) Etanercept 6 (11,8%) MCTD 3 (5,9%) TNF inhibitors 4 (7,8%) RA 12(23,5%) Tocilizumab 2 (3,9%) IPAF 4 (7,8%) Azathioprine 10 (19,6%) Leflunomide 3 (5,9%) MP pulses 15 (29,4%) Rituximab 12 (23,5%) Abatacept 5 (9,8%) Tacrolimus 5 (9,8%) Antibodies - n (% ) Anti-myositis 6 (11,8%) Anti-sclerosis 15(29,4%) Anti- Ro 25 (49%) Anti- RNP 3 (5,9%) ANA 35(68,6%) RF 21(41,2%) Anti- Synt 6 (11,8%) Note . POCD = Pulmonary Obstructive Chronic Disease SLE = Systemic Lupus Erythematosus MCTD = Mixed Connective Tissue Disease RA = Rheumatoid Arthritis IPAF = Insterstitial Pneumonia with Autoimmune Features Anti RNP = Anti RiboNucleoProtein ANA = Antinuclear Antibodies RF = Rheumatoid Factor Anti Synt =Anti-synthetase PHT= Pulmonary Hypertension UIP = Usual Interstitial Pneumonia Incons-UIP = Inconsistent Usual Interstitial PneumoniaNSIP = Nonspecific Interstitial PneumoniaFib-NSIP = Fibrosing Nonspecific Interstitial PneumoniaOP = Organizing PneumoniaLIP = Lymphoid Interstitial PneumoniaTNF inhibitor = Tumor Necrosis Factor inhibitorMP = Metyhprednisolone Figure 1. During follow up, 1 patient with sarcoidosis died of COVID19 bilateral pneumonia. Conclusion In our series most patients were middle aged women. Anti-Ro antibodies and smoking status (former or current) were common among patients. Common clinical features were Raynaud (45%), skin affection (45%) and arthritis (40%). 47% of the patients expressed dyspnea at ILD diagnosis. 29,4% were treated with MP pulses, 23,5% with rituximab, 31,4% with mycofenolate mophetil. Fibrosing pattern in HRCT (UIP and fib-NSIP) was the most prevalent. 20% of the patients had progressive fibrosis under PFT criteria and 18% under HCRT. More studies of ILD-CTD are necessary to identify factors for progression and response to treatment and throw out more conclusions of prediction and prognosis of disease. Disclosure of Interests None declared